Department of Gastroenterology and Hepatology, Kusunoki Hospital, Japan.
Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, Japan.
Intern Med. 2022 Mar 15;61(6):841-849. doi: 10.2169/internalmedicine.8290-21. Epub 2021 Sep 4.
We encountered a 47-year-old woman with polycystic liver disease (PLD) and severe malnutrition successfully treated by living-donor liver transplantation (LDLT). Her PLD became symptomatic with abdominal distension and appetite loss. Transcatheter arterial embolization and percutaneous cyst drainage failed to improve her symptoms. ABO-incompatible LDLT from her husband was performed after rituximab administration and mycophenolate mofetil introduction. Although she showed severe postoperative complications, she ultimately regained the ability to walk and was discharged. Because advanced PLD cases are difficult to treat conservatively or with surgery, like fenestration and hepatectomy, liver transplantation should be considered before it becomes too late.
我们成功治疗了一位 47 岁患有多囊肝病（PLD）和严重营养不良的女性患者，她接受了活体供肝肝移植（LDLT）。她的 PLD 出现了腹部肿胀和食欲不振等症状。经导管动脉栓塞和经皮囊肿引流未能改善她的症状。在给予利妥昔单抗和霉酚酸酯后，她接受了来自丈夫的 ABO 不相容 LDLT。尽管她出现了严重的术后并发症，但最终恢复了行走能力并出院。由于晚期 PLD 病例很难通过保守治疗或手术（如开窗和肝切除术）治疗，因此在为时过晚之前应考虑进行肝移植。
